Myelofibrosis is a clonal disorder arising from the neoplastic transformation of early hematopoietic stem cells. Primary myelofibrosis is categorized as a chronic myeloproliferative disorder, and secondary myelofibrosis can occur in later stages of the other myeloproliferative neoplasms such as chronic myelogenous leukemia, polycythemia vera, and essential thrombocytosis. The disorder is characterized by anemia, extramedullary hematopoiesis, leukoerythroblastosis, teardrop-shaped red blood cells in peripheral blood, and hepatosplenomegaly.
The annual incidence of primary myelofibrosis is approximately 0.5-1.5 cases per 100,000 individuals, although an increased prevalence is noted in Ashkenazi Jews. It is a progressive disease, and median overall survival is 6-7 years.
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Cite this: Anand Patel. Fast Five Quiz: Myelofibrosis Management - Medscape - Jun 20, 2023.